Juvenile xanthogranuloma with recurrent subdural effusions.

Area of Science:

• Pediatric Dermatology
• Neurology
• Oncology

Background:

• Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder.
• JXG typically presents as benign cutaneous lesions.
• Systemic involvement in JXG is uncommon but can occur.

Observation:

• A 2-year-old girl presented with extensive cutaneous xanthogranuloma.
• The patient experienced recurrent subdural effusions.
• Diagnostic challenges included differentiating JXG from histiocytosis X.

Findings:

• Light and electron microscopy of skin and cerebrospinal fluid cells were crucial for diagnosis.
• Recurrent subdural effusions showed a positive response to cranial irradiation.
• Microscopic findings confirmed juvenile xanthogranuloma.

Implications:

• This case highlights the importance of thorough investigation in atypical JXG presentations.
• Cranial irradiation may be an effective treatment for subdural effusions associated with JXG.
• A spectrum of proliferative changes, including systemic involvement, should be considered in JXG.